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Cochlear implants, hearing loss

Poornapriya Ramamurthy, Joshua B White, Joong Yull Park, Richard I Hume, Fumi Ebisu, Flor Mendez, Shuichi Takayama, Kate F Barald
BACKGROUND: To send meaningful information to the brain, an inner ear cochlear implant (CI) must become closely coupled to as large and healthy a population of remaining Spiral Ganglion Neurons (SGN) as possible. Inner ear gangliogenesis depends on macrophage migration inhibitory factor (MIF), a directionally attractant neurotrophic cytokine made by both Schwann and supporting cells (Bank et al., 2012). MIF-induced mouse embryonic stem cell (mESC)-derived "neurons" could potentially substitute for lost or damaged SGN...
October 19, 2016: Developmental Dynamics: An Official Publication of the American Association of Anatomists
Juul Verberne, Frank Risis, Luke Campbell, Scott Chambers, Stephen O'Leary
HYPOTHESIS: Scala tympani morphology influences the insertion dynamics and intra-scalar position of straight electrode arrays. BACKGROUND: Hearing preservation is the goal of cochlear implantation with current thin straight electrode arrays. These hug the lateral wall, facilitating full, atraumatic insertions. However, most studies still report some postoperative hearing loss. This study explores the influence of scala tympani morphology on array position relative to the basilar membrane and its possible contribution to postoperative hearing loss...
October 12, 2016: Otology & Neurotology
Stephanie Kletke, Vaishnavi Batmanabane, Tianyang Dai, Ajoy Vincent, Shuning Li, Karen A Gordon, Blake C Papsin, Sharon L Cushing, Elise Héon
The co-occurrence of hearing impairment and visual dysfunction is devastating. Most deaf-blind etiologies are genetically determined, the commonest being Usher syndrome (USH). While studies of the congenitally deaf population reveal a variable degree of visual problems, there are no effective ophthalmic screening guidelines. We hypothesized that children with congenital sensorineural hearing loss (SNHL) and vestibular impairment were at an increased risk of having Usher syndrome. A retrospective chart review of 33 cochlear implants recipients for severe to profound SNHL and measured vestibular dysfunction was done to determine the ocular phenotype...
October 15, 2016: Clinical Genetics
Claudia Frick, Marcus Müller, Ute Wank, Anke Tropitzsch, Benedikt Kramer, Pascal Senn, Helge Rask-Anderson, Karl-Heinz Wiesmüller, Hubert Löwenheim
Cochlear implants (CI) allow for hearing rehabilitation in patients with sensorineural hearing loss or deafness. Restricted CI performance results from the spatial gap between spiral ganglion neurons and the CI, causing current spread that limits spatially restricted stimulation and impairs frequency resolution. This may be substantially improved by guiding peripheral processes of spiral ganglion neurons towards and onto the CI electrode contacts. An injectable, peptide-based hydrogel was developed which may provide a permissive scaffold to facilitate neurite growth towards the CI...
October 4, 2016: Colloids and Surfaces. B, Biointerfaces
Ji Eun Choi, Sung Hwa Hong, Jong Ho Won, Hee-Sung Park, Young Sang Cho, Won-Ho Chung, Yang-Sun Cho, Il Joon Moon
Adults who score 50% correct or less in open-set sentence recognition test under the best aided listening condition may be considered as candidates for cochlear implant (CI). However, the requirement for 'the best aided listening condition' needs significant time and clinical resources to ensure such condition. As speech signals are composed of dynamic spectral and temporal modulations, psychoacoustic sensitivity to the combinations of spectral and temporal modulation cues may be a strong predictor for aided speech recognition...
October 12, 2016: Scientific Reports
Douglas P Sladen, Matthew L Carlson, Brittany P Dowling, Amy P Olund, Kathryn Teece, Melissa D DeJong, Alyce Breneman, Ann Peterson, Charles W Beatty, Brian A Neff, Colin L Driscoll
OBJECTIVES/HYPOTHESIS: This study was designed to examine speech recognition and self-perceived health-related quality of life (HRQoL) received from cochlear implantation among a cohort of adults and children with a short duration of unilateral hearing loss greater than 6 months, but less than 2 years. STUDY DESIGN: Single-subject repeated measures prospective study. METHODS: This study assessed changes in speech recognition and self-perceived quality of life by prospectively analyzing data at the preoperative evaluation and at the 3-month and 6-month postactivation intervals...
October 12, 2016: Laryngoscope
Mo Chen, Zhaoyan Wang, Zhiwen Zhang, Xun Li, Weijing Wu, Dinghua Xie, Zi-An Xiao
OBJECTIVE: The present study aims to test whether deaf children with unilateral cochlear implantation (CI) have higher intelligence quotients (IQ). We also try to find out the predictive factors of intelligence development in deaf children with CI. METHODS: Totally, 186 children were enrolled into this study. They were divided into 3 groups: CI group (N = 66), hearing loss group (N = 54) and normal hearing group (N = 66). All children took the Hiskey-Nebraska Test of Learning Aptitude to assess the IQ...
November 2016: International Journal of Pediatric Otorhinolaryngology
Katrien Vermeire, Leslie Wexler, Andrea Vambutas
We present a 3-year old boy with Leopard syndrome. His clinical manifestations included a congenital bilateral sensorineural hearing loss. He underwent cochlear implantation on the right side at age 1 year and on the left side at age 1.5 years. The patient is doing very well and mainstreamed in a regular pre-school program with a teacher of the deaf and home based speech therapy. Bilateral cochlear implantation in the case of a child with Leopard syndrome can be successful.
November 2016: International Journal of Pediatric Otorhinolaryngology
Soren Gantt, Francois Dionne, Fred K Kozak, Oran Goshen, David M Goldfarb, Albert H Park, Suresh B Boppana, Karen Fowler
Importance: Congenital cytomegalovirus (cCMV) infection is a major cause of childhood deafness. Most cCMV infections are not diagnosed without newborn screening, resulting in missed opportunities for directed care. Objective: To estimate the cost-effectiveness of universal and targeted newborn cCMV screening programs compared with no cCMV screening. Design, Setting, and Participants: Models were constructed using rates and outcomes from prospective cohort studies of newborn cCMV screening in US postpartum care and early hearing programs...
October 10, 2016: JAMA Pediatrics
Adem Binnetoğlu, Tekin Bağlam, Murat Sarı, Yavuz Gündoğdu, Çağlar Batman
Duplication of the internal auditory canal is an uncommon, congenital malformation that can be associated with sensorineural hearing loss owing to aplasia/hypoplasia of the vestibulocochlear nerve. Only 14 such cases have been reported to date. We report the case of a 13-month-old girl with bilateral, congenital, sensorineural hearing loss caused by narrow, duplicated internal auditory canals and discuss the challenges encountered in the diagnosis and treatment of this condition.
August 2016: Journal of International Advanced Otology
Hasan Demirhan, Muhammet Yıldız, Özgür Yiğit
OBJECTIVE: The objective of this study was to explore the usefulness of 1000-Hz tone burst (TB) stimuli for detecting cervical vestibular-evoked myogenic potential (cVEMP) abnormalities in patients with a cochlear implant (CI). MATERIALS AND METHODS: Thirty asymptomatic patients who received unilateral CI because of severe bilateral sensorineural hearing loss were assessed for cVEMP produced by TB stimuli at two frequencies (500 and 1000 Hz) in the airway. VEMPs were recorded when the devices were switched to the on (CI-on) and off (CI-off) positions...
August 2016: Journal of International Advanced Otology
Beier Qi, Ziye Liu, Xin Gu, Bo Liu
CONCLUSION: Mandarin-speaking adults can use the Fine Structure Processing (FSP) coding strategy as well as the Continuous Interleaved Sampling (CIS+) coding strategy. No loss in performance was observed after switch-over. Tone identification improves over time with the FSP coding strategy, which is of benefit to tonal-language users. After some time, fine structure was preferred. OBJECTIVE: This study aimed to determine speech perception, tone perception, and the subjective preferences of Mandarin-speaking adults who received the FSP coding strategy, at upgrade from the CIS + coding strategy...
October 5, 2016: Acta Oto-laryngologica
Friedrich Ihler, Jenny Blum, Max-Ulrich Berger, Bernhard G Weiss, Christian Welz, Martin Canis
Semi-implantable transcutaneous bone conduction devices are treatment options for conductive and mixed hearing loss (CHL/MHL). For counseling of patients, realistic simulation of the functional result is desirable. This study compared speech recognition in noise with a semi-implantable transcutaneous bone conduction device to external stimulation with a bone conduction device fixed by a headband. Eight German-language adult patients were enrolled after a semi-implantable transcutaneous bone conduction device (Bonebridge, Med-El) was implanted and fitted...
October 3, 2016: Trends in Hearing
Laura S M Derks, Inge Wegner, Adriana L Smit, Hans G X M Thomeer, Vedat Topsakal, Wilko Grolman
INTRODUCTION: Cochlear implantation is an increasingly common procedure in the treatment of severe to profound sensorineural hearing loss (SNHL) in children and adults. It is often performed as a day-case procedure. The major drive towards day-case surgery has been from a logistical, economical and societal perspective, but we also speculate that the patient's quality of life (QoL) is at least equal to inpatient surgery if not increased as a result of rapid discharge and rehabilitation...
October 3, 2016: BMJ Open
Hannah J D North, Deborah Mawman, Martin O'Driscoll, Simon R Freeman, Scott A Rutherford, Andrew T King, Charlotte Hammerbeck-Ward, D Gareth Evans, Simon K W Lloyd
In neurofibromatosis type 2 (NF2) bilateral vestibular schwannomas (VS) or their treatment usually results in bilateral hearing loss. Cochlear implantation (CI) was traditionally not used in these patients due to concern that retrocochlear disease would render the implant ineffective. This paper describes the auditory outcomes of CI in 13 patients with NF2 and includes patients with untreated VS and patients undergoing VS removal with cochlear nerve preservation. The non-user rate was 7.7%. Of the active users, median CUNY score was 98%, median BKB score in quiet was 90% and median BKB score in noise was 68%...
July 2016: Cochlear Implants International
Anu Sharma, Hannah Glick, Emily Deeves, Erin Duncan
We review evidence for a high degree of neuroplasticity of the central auditory pathways in early childhood, citing evidence of studies of the P1 and N1 cortical auditory evoked potentials in congenitally deaf children receiving cochlear implants at different ages during childhood, children with auditory neuropathy spectrum disorder and children with hearing loss and comorbid multiple disabilities. We discuss neuroplasticity, including cortico-cortical de-coupling and cross-modal re-organization that occurs in deafness...
December 2015: Otorinolaringologia
Joshua J Gnanasegaram, William J Parkes, Sharon L Cushing, Carmen L McKnight, Blake C Papsin, Karen A Gordon
Vestibular end organ impairment is highly prevalent in children who have sensorineural hearing loss (SNHL) rehabilitated with cochlear implants (CIs). As a result, spatial perception is likely to be impacted in this population. Of particular interest is the perception of visual vertical because it reflects a perceptual tilt in the roll axis and is sensitive to an imbalance in otolith function. The objectives of the present study were thus to identify abnormalities in perception of the vertical plane in children with SNHL and determine whether such abnormalities could be resolved with stimulation from the CI...
2016: Frontiers in Integrative Neuroscience
Abir Oueida El Sadik, Mohamed Hafez Shaaban
BACKGROUND: Internal auditory canal (IAC) stenosis and vestibulocochlear nerve abnormalities have been reported to be associated with sensorineural hearing loss (SNHL). Previous studies classified the normal dimensions of the IAC and its anomalies with no consideration of the vestibulochoclear nerve. Other studies categorized the vestibulocochlear nerve development in only stenotic canals. In the present study, an anatomical classification of the normal dimensions of the IAC and its anomalies and their association with malformations of the vestibulocochlear nerve and its subdivisions were described...
September 26, 2016: Folia Morphologica (Warsz)
Satu Turunen-Taheri, Åsa Skagerstrand, Sten Hellström, Per-Inge Carlsson
CONCLUSIONS: Patients with severe vision impairment in combination with severe-to-profound hearing loss seem to have a higher risk for effects on QoL, including: mobility, the ability to provide self-care and perform usual activities, and levels of anxiety and depression, compared with patients with only severe-to-profound hearing loss. OBJECTIVES: To study the quality-of-life (QoL) and audiological rehabilitation of the severely vision-impaired patient population among adults with severe-to-profound hearing loss in Sweden...
September 23, 2016: Acta Oto-laryngologica
J M Espinosa-Sanchez, J A Lopez-Escamez
Menière's disease (MD) is a chronic multifactorial disorder of the inner ear characterized by episodic vestibular symptoms associated with sensorineural hearing loss, tinnitus, and aural pressure. Epidemiologic and genomic evidence supports a genetic susceptibility with multiple biochemical pathways involved, including the endocrine system, innate immune response, and autonomic nervous system. Allergens, infectious agents, vascular events, or genetic factors could modify inner-ear homeostasis and trigger MD...
2016: Handbook of Clinical Neurology
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